Lymphatic Disorder Management in Pediatric Patients With Congenital Heart Disease in European Pediatric Cardiology Centers : Current Status, Disparities, and Future Considerations
(2024) In Journal of the American Heart Association 13(22).- Abstract
BACKGROUND: Lymphatic disorders such as protein-losing enteropathy, plastic bronchitis, and chylothorax are important complications of the Fontan circulation and ultimately result in an increased risk of early death. Several European centers are now performing lymphatic procedures. The aim of this study is to map the extent of these lymphatic disorders and treatments provided across European pediatric cardiology centers. METHODS AND RESULTS: A survey was circulated to 49 European pediatric cardiology centers consisting of 37 questions including a mix of binary, categorical, and continuous variables. Thirty-one centers (63%) participated in the study, performing a median of 250 (interquartile range, 178-313) cardiopulmonary bypass... (More)
BACKGROUND: Lymphatic disorders such as protein-losing enteropathy, plastic bronchitis, and chylothorax are important complications of the Fontan circulation and ultimately result in an increased risk of early death. Several European centers are now performing lymphatic procedures. The aim of this study is to map the extent of these lymphatic disorders and treatments provided across European pediatric cardiology centers. METHODS AND RESULTS: A survey was circulated to 49 European pediatric cardiology centers consisting of 37 questions including a mix of binary, categorical, and continuous variables. Thirty-one centers (63%) participated in the study, performing a median of 250 (interquartile range, 178-313) cardiopulmonary bypass procedures per year. Chylothorax emerged as the most prevalent lymphatic disorder followed by protein-losing enteropathy and plastic bronchitis. The most common diagnostic investigation method was noncontrast magnetic resonance lymphangiography (52%). Eleven centers (35%) conducted lymphatic interventions with a median of 3 (interquartile range, 1-4) procedures per year and 12 (interquartile range, 5-15) interventions in total per center. CONCLUSIONS: This study confirms the rarity of and variation in treatment approaches for lymphatic disorders across Europe. With at least 11 centers offering lymphatic interventions, the adoption of these procedures is on the rise in Europe. To improve the quality of care and treatment outcomes for these complex patients, it is crucial to consider evidence-based lymphatic diagnostics, interventional lymphatic procedures, and the centralization of services in Europe.
(Less)
- author
- Safi, Sanam
; Hjortdal, Vibeke E.
; Gewillig, Marc
; Miller, Owen
; Abumehdi, Mohammad Ryan
; Cantinotti, Massimiliano
; Grotenhuis, Heynric
; Raimondi, Francesca
; Garrido, Almudena Ortiz
; Roest, Arno
; Sunnegårdh, Jan
; Saleats, Thomas
; Brun, Henrik
; Pärna, Helen
; Nolan, Oscar
; Rotés, Anna Sabaté
; Deri, Antigoni
; Wolf, Daniel De
LU
; Herberg, Ulrike
; Liuba, Petru
LU
; Möller, Thomas
; Óskarsson, Gylfi
LU
; Rebonato, Micol
; Helbing, Willem A.
; Doros, Gabriela
; Muntean, Iolanda
; Ojala, Tiina
; Lubaua, Inguna
; Bhat, Misha
LU
; Wacker, Julie
; Bonello, Beatrice
; Ramcharan, Tristan
; Greil, Gerald
; Sendzikaite, Skaiste
; Bonnet, Damien
; Marek, Jan
; Voges, Inga
and McMahon, Colin J.
(Less) - organization
- publishing date
- 2024-11-19
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- congenital heart disease, imaging modalities, lymphatic disorders, lymphatic interventions, MRI
- in
- Journal of the American Heart Association
- volume
- 13
- issue
- 22
- article number
- e036597
- publisher
- Wiley-Blackwell
- external identifiers
-
- pmid:39508150
- scopus:85210105802
- ISSN
- 2047-9980
- DOI
- 10.1161/JAHA.124.036597
- language
- English
- LU publication?
- yes
- additional info
- Publisher Copyright: © 2024 The Author(s).
- id
- 1f6f4f56-fe83-4926-95dc-ebc5976ad981
- date added to LUP
- 2025-01-09 11:15:17
- date last changed
- 2025-07-11 02:43:06
@article{1f6f4f56-fe83-4926-95dc-ebc5976ad981,
abstract = {{<p>BACKGROUND: Lymphatic disorders such as protein-losing enteropathy, plastic bronchitis, and chylothorax are important complications of the Fontan circulation and ultimately result in an increased risk of early death. Several European centers are now performing lymphatic procedures. The aim of this study is to map the extent of these lymphatic disorders and treatments provided across European pediatric cardiology centers. METHODS AND RESULTS: A survey was circulated to 49 European pediatric cardiology centers consisting of 37 questions including a mix of binary, categorical, and continuous variables. Thirty-one centers (63%) participated in the study, performing a median of 250 (interquartile range, 178-313) cardiopulmonary bypass procedures per year. Chylothorax emerged as the most prevalent lymphatic disorder followed by protein-losing enteropathy and plastic bronchitis. The most common diagnostic investigation method was noncontrast magnetic resonance lymphangiography (52%). Eleven centers (35%) conducted lymphatic interventions with a median of 3 (interquartile range, 1-4) procedures per year and 12 (interquartile range, 5-15) interventions in total per center. CONCLUSIONS: This study confirms the rarity of and variation in treatment approaches for lymphatic disorders across Europe. With at least 11 centers offering lymphatic interventions, the adoption of these procedures is on the rise in Europe. To improve the quality of care and treatment outcomes for these complex patients, it is crucial to consider evidence-based lymphatic diagnostics, interventional lymphatic procedures, and the centralization of services in Europe.</p>}},
author = {{Safi, Sanam and Hjortdal, Vibeke E. and Gewillig, Marc and Miller, Owen and Abumehdi, Mohammad Ryan and Cantinotti, Massimiliano and Grotenhuis, Heynric and Raimondi, Francesca and Garrido, Almudena Ortiz and Roest, Arno and Sunnegårdh, Jan and Saleats, Thomas and Brun, Henrik and Pärna, Helen and Nolan, Oscar and Rotés, Anna Sabaté and Deri, Antigoni and Wolf, Daniel De and Herberg, Ulrike and Liuba, Petru and Möller, Thomas and Óskarsson, Gylfi and Rebonato, Micol and Helbing, Willem A. and Doros, Gabriela and Muntean, Iolanda and Ojala, Tiina and Lubaua, Inguna and Bhat, Misha and Wacker, Julie and Bonello, Beatrice and Ramcharan, Tristan and Greil, Gerald and Sendzikaite, Skaiste and Bonnet, Damien and Marek, Jan and Voges, Inga and McMahon, Colin J.}},
issn = {{2047-9980}},
keywords = {{congenital heart disease; imaging modalities; lymphatic disorders; lymphatic interventions; MRI}},
language = {{eng}},
month = {{11}},
number = {{22}},
publisher = {{Wiley-Blackwell}},
series = {{Journal of the American Heart Association}},
title = {{Lymphatic Disorder Management in Pediatric Patients With Congenital Heart Disease in European Pediatric Cardiology Centers : Current Status, Disparities, and Future Considerations}},
url = {{http://dx.doi.org/10.1161/JAHA.124.036597}},
doi = {{10.1161/JAHA.124.036597}},
volume = {{13}},
year = {{2024}},
}